Pheochromocytoma

A 40-year-old gentleman presents with episodic headaches and palpitations. Attributing it to his tendency to worry excessively, he put it off for several months until he began to sweat episodically too. In the clinic, his blood pressure is found to be elevated at 160/120 mmHg. Plasma metanephrines are elevated and an abdominal CT scan reveals an adrenal mass.

Introduction

Nonmalignant tumor of adrenal medulla

most common adrenal tumor in adults (vs neuroblastoma, most common in children)

secretes catecholamines causing episodic hypertension

Pathogenesis

from chromaffin cells of neural crest origin

Epidemiology

40-50 years old

Rule of 10’s

10% malignant

10% bilateral

10% kids

10% extra-adrenal

bladder, organ of Zuckerkandl (bifurcation of aorta)

10% calcify

Associated conditions

von Hippel-Lindau disease

MEN 2A

MEN 2B

neurofibromatosis type 1

Presentation

Symptoms are episodic

5 P’s

Pressure (↑ BP) Pain (headache)

Perspiration

Palpitations (tachycardia)

Pallor

mediated by tumor secretion of epinephrine, norepinephrine, and dopamine

(M1.EC.4713)
A 34-year-old woman comes to the emergency department complaining of severe headache and anxiety, diaphoresis, and palpitations for the last 30 minutes. She has had several similar episodes over the past few weeks. She has no significant past medical history and has a 10 pack-year smoking history. She takes no illicit drugs. Her blood pressure on arrival is 181/80 mmHg and her pulse is 134/min. If this patient was given metoprolol, how would her blood pressure respond?
Review Topic

(M1.EC.64)
A 34-year-old female presents to the emergency room with headache and palpitations. She is sweating profusely and appears tremulous on exam. Vital signs are as follows: HR 120, BP 190/110, RR 18, O2 99% on room air, and Temp 37C. Urinary metanephrines and catechols are positive. Which of the following medical regimens is contraindicated as a first-line therapy in this patient?
Review Topic

(M1.EC.110)
A 40-year-old female presents with palpitations, headache, and episodic diaphoresis. On physical exam she has a blood pressure of 170/90 mmHg. Urine analysis shows elevated levels of vanillylmandelic acid. Figure A shows the histology of the adrenal gland. Hyperactivity of cells in which of the follow regions is likely responsible for this patient's symptoms?
Review Topic

(M1.EC.116)
A 49 year-old-male presents with a primary complaint of several recent episodes of severe headache, sudden anxiety, and a "racing heart". The patient originally attributed these symptoms to stress at work; however, these episodes are becoming more frequent and severe. Laboratory evaluation during such an episode reveals elevated plasma free metanephrines. Which of the following additional findings in this patient is most likely?
Review Topic